An Important Health Message to Healthcare Professionals

You provide information to parents about their This program is designed to inform caregivers and
children’s health, and in turn, parents trust you as a parents about PI – what it is and how it is detected –
reliable health source. in order to raise awareness of PI so that it does not
continue to go undiagnosed in children.
If there are children in your care who are sick often,
ask yourself the following questions: Included with this program are:

? Do the children you see frequently exhibit recurring
illnesses without explanation?
? Are you familiar with Primary Immunodeficiency (PI)?
? Do you think families are aware of PI?
PI is a genetic defect in the immune system that does
not allow the body to fight off germs and viruses as
quickly as a person with a healthy immune system. To
date, there are more than 150 identified forms of PI
that greatly range in severity. PI is characterized by
chronic, recurring, persistent and/or debilitating
illnesses that in severe cases, can be fatal.
PI affects more than 10 million people around the world,
including 1,000,000 in the U.S. But how many people
know about it? Not enough.
To help you provide parents with information on health
issues facing children today, the Jeffrey Modell
Foundation (JMF) and Weekly Reader Custom Publishing
have collaborated to bring you this free, educational
program, What is Primary Immunodeficiency (PI)?
t four reproducible informational panels, including:
. this letter
. a reproducible letter to parents/guardians
. a reproducible informational page about PI
. a reproducible children’s activity page
t a poster of the immune system to hang in an area
visible to children to use as a teaching tool (the
poster is located on the reverse side of these
information panels)
Please photocopy and distribute the information about
PI to families and colleagues. Please feel free to
photocopy the activity page to distribute to children
to complete on their own, or send it home with the
parents’ information for children to do with their
families. If you suspect the presence of a PI disorder
in a child you know, urge the family to visit the JMF
Website at www.info4pi.org.

If you have children who you see regularly, who are continuously afflicted with chronic ailments and
take longer to recuperate from repeated illnesses, please do not rule out PI as a possibility for the
child’s recurring health problems. If parents know about PI and are educated about it, they can take
action. Once informed, they can speak to their child’s pediatrician about PI and will be able to rule it
out as a possibility, or get help for an affected child.
Thank you for taking the time to learn about PI and educating others about it. For more information,
please visit the Website for the Jeffrey Modell Foundation at www.info4pi.org.
Sincerely,
nd Jeffrey Modell Foundation & Weekly Reader Custom Publishing
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If you or a loved one has recently been diagnosed
with PI, please contact the Jeffrey Modell Foundation © 2008 Lifetime Learning Systems®, Inc. and
at info@jmfworld.org for a free patient starter kit. Weekly Reader Custom Publishing. Created
for the Jeffrey Modell Foundation.
 An Important Health Message to Parents/Guardians

Dear Parents/Guardians,
Primary Immunodeficiency (PI)
What you want most for your loved ones is for them to be happy
causes children and adults to
and healthy. As a parent, you do what you can to make sure your have infections that come back
child is feeling well, and you take care of your child when he/she is ill. frequently, or are unusually hard
to cure. If you or someone you
When it comes to your child’s health, the more knowledge you know is affected by two or more of
have, the more you can help your child. That is why the Jeffrey the following warning signs, speak
to a physician about the possible
Modell Foundation (JMF), in conjunction with Weekly Reader Custom presence of an underlying Primary
Publishing, developed What is Primary Immunodeficiency (PI)?, a Immunodeficiency.
program designed to inform parents about Primary Immunodeficiency
1) Eight (8) or more new ear
(PI), including what it is and how to detect it. infections within one (1) year.
PI is a genetic defect in the immune system. This genetic flaw does
2) Two (2) or more serious sinus
not allow the body to fight off germs and viruses as quickly or as infections within one (1) year.
regularly as a person with a healthy immune system. So far, there
are more than 150 identified forms of PI that vary in severity. PI is 3) Two (2) or more months on
antibiotics with little effect.
characterized by chronic, recurring, persistent and/or debilitating
illnesses that, in severe cases, can be fatal. At least one in every 4) Two
 (2) or more bouts of
500 Americans is diagnosed with PI; however, doctors estimate that pneumonia within one (1) year.
PI goes undiagnosed in an additional one in every 500 Americans. 5) Failure of an infant to gain
weight and/or grow normally.
Here are some additional facts about PI:
t P
I symptoms sometimes appear as a common infection (often 6) Recurrent
 deep skin or organ
of the sinuses, ears or lungs), a gastrointestinal problem or abscesses.
inflammation of the joints. 7) Persistent
 thrush in mouth
or elsewhere on skin, after
t I
nfections can be chronic, coming back often and with more age one (1).
severity, requiring more time for recovery.
8) Need
 for intravenous anti-
tM
ales and females of all ages are at risk of having PI, since it biotics to clear infections.
is a genetic flaw in the immune system.
9) Two
 (2) or more
t E
arly diagnosis and treatment are necessary to prevent deep-seated
infections.
recurring infections from inflicting permanent damage.
10) A
 family history
t P
I should not be confused with AIDS. PI is genetic, meaning
of Primary
the immune system’s flaw is built into a person’s DNA. AIDS Immunodeficiency.
is acquired by becoming infected with the HIV virus.

If your child exhibits any of these symptoms on a frequent basis,

contact your pediatrician and ask him/her to rule out PI as a
possibility. PI can be diagnosed with a blood test. Diagnosis and If you or a loved one has
treatment begin with awareness. recently been diagnosed
with PI, please contact the
Visit the JMF Website at www.info4pi.org for more information Jeffrey Modell Foundation
about PI. You will find that it is an interesting and valuable resource at info@jmfworld.org for a
for information. free patient starter kit.

Sincerely,
Jeffrey Modell Foundation & Weekly Reader Custom Publishing
© 2008 Lifetime Learning Systems®, Inc. and
Weekly Reader Custom Publishing. Created
for the Jeffrey Modell Foundation.

Primary Immunodeficiency (PI) occurs when one or more essential parts of the immune system does not
work correctly. People with PI cannot get rid of germs or protect themselves from germs as well as they
should, causing frequent infections and other problems that are hard to cure. Infections may appear to be
common illnesses such as sinus and ear infections, pneumonia, fever, common colds and bronchitis.
If you or someone you know is
affected by two or more of the
following warning signs, speak
to a physician about the possible
presence of an underlying
Primary Immunodeficiency.
1) Eight (8) or more new
ear infections within one
(1) year.
2) Two (2) or more serious
sinus infections within one
(1) year.
3) Two (2) or more months on
antibiotics with little effect.
4) Two
 (2) or more bouts
of pneumonia within one
(1) year.
5) Failure of an infant to
gain weight and/or grow
normally.
6) Recurrent deep skin or
organ abscesses.
7) Persistent
 thrush in mouth
or elsewhere on skin, after
age one (1).
8) Need
 for intravenous
antibiotics to clear
infections.
9) Two (2) or more deep-seated
infections.
10) A family history of Primary
Immunodeficiency.
An Important Health Message for Everyone
macrophage lymphocyte phagocyte
How is PI diagnosed?
PI can be diagnosed with a blood test. A blood test will show the genetic
makeup of the immune system and whether or not any parts are missing
or not working correctly. An immune specialist, called an immunologist,
might also choose to test your child’s immune system with a series of
vaccines to see how your child’s body responds to the shots. A physical
exam and an evaluation of family medical history may also be part of the
path to diagnosis.
How is PI treated?
With more than 150 different types of PI, there are also different types
of treatments. The earlier the detection of PI, the better. Some recurring
infections can be treated with antibiotics to help prevent long-term
damage and improve the patient’s quality of life.
Another alternative for treatment is antibody replacement therapy, also
known as IVIG therapy. It works by replacing the antibodies (proteins that
help white blood cells fight and destroy germs) that the body isn’t able to
make on its own.
In other cases, bone marrow transplants, enzyme replacement or gene
therapy may be appropriate.
What is the Jeffrey Modell Foundation?
The Jeffrey Modell Foundation (JMF) is a global non-profit organization
established in Jeffrey’s memory by his parents, Vicki and Fred Modell.
Jeffrey struggled with a form of PI for years, before succumbing to
pneumonia in 1986 at the age of 15. The Foundation is dedicated to
early and precise diagnosis, meaningful treatments and ultimately, cures
for Primary Immunodeficiencies. Today, JMF focuses its efforts in research,
physician education, patient support, public awareness and advocacy.
The Jeffrey Modell Foundation believes that with treatment intervention,
patients with PI will be able to gain or maintain control of their lives by:
. participating in work, school, family and social activities;
. decreasing the number and severity of infections;
. having few, if any, side effects from medications and
other treatments; and
. feeling good about themselves and their treatment program.
Visit the JMF Website at www.info4pi.org
for more information about PI.
© 2008 Lifetime Learning Systems®, Inc. and Weekly Reader Custom Publishing.
Created for the Jeffrey Modell Foundation.
 Children’s Activity Page

infections, germs,
Every day, your body fights off
troying invaders,
bacteria and parasites. It is des
of its number
called pathogens, with the help
tem. The immune
one defense: the immune sys
y healthy. Match the words below with their definitions.
system’s job is to keep your bod
learn more about
Finish the activities below to 1. Immune system _______ 4. Antibody _______
tem . The answers are at
your body’s immune sys
the bottom of the page. 2. Fever _______ 5. Pathogen _______

Test your knowledge about your immune
system by determining if each statement is
true or false. Check off your answer.
1) Blood cells are produced inside your bones
in the bone marrow.
oTrue oFalse
2) Germs and viruses trigger your red blood
cells into defensive mode.
oTrue oFalse
3) Some types of bacteria live inside your body
and help protect it from germs.
oTrue oFalse
4) Your thymus, the gland where some white
blood cells go to become T-cells, shrinks in
size as you get older.
oTrue oFalse
For more games and activities,
visit www.info4pi.org and click
3. Tonsils _______
A) any foreign invader found in your body, such as a
germ, virus or bacteria
B) located in the back of your mouth near your
throat, they protect the entrance to your
respiratory and digestive systems by
destroying bacteria
C) the cells and organs that work together to keep
your body healthy and fight off germs, bacteria,
viruses and infections
D) a protein that white blood cells use to mark virus
cells so that other white blood cells will know to
attack the virus
E) a body temperature higher than 98.6 degrees
Fahrenheit – sometimes when a virus is detected,
your white blood cells will tell your brain to raise
your body’s temperature, making it too hot for the
virus to multiply
bone marrow

5) White blood cells sometimes attack on KIDS Korner.

spleen, thymus, tonsils, lymph nodes,
Immune System Order:
healthy cells within your body by mistake. 1. C, 2. E, 3. B, 4. D, 5. A

oTrue oFalse Finding Out About the Immune System:

mune disease.
tissue. When this happens, it’s called an autoim-
healthy cells within your body, destroying healthy
5. True. Sometimes white blood cells attack
together.

The words below are scrambled. Put the letters in the correct order to find decreases to the size of two grapes joined

out the parts of the immune system.

the size of a lemon, but as you get older, it
4. True. Your thymus is large at birth, about
harmful bacteria from reproducing.

pelsen htmsuy slintos within your stomach and skin that helps to stop
3. True. There is harmless bacteria that lives
blood cells into action.
Germs, viruses and bacteria trigger white

___________________ ___________________ ___________________ out your body and remove carbon dioxide.
2. False. Red blood cells carry oxygen through-
blood cells are made.

Think About This!

the core of your bones where red and white
1. True. Bone marrow is the substance within

ylhpm oedsn beon rarwom How many words can The Truth About Your Immune System:

you make using the Answers:

letters in the phrase

___________________ ___________________ “immune system”?
© 2008 Lifetime Learning Systems®, Inc. and Weekly Reader Custom Publishing.
Created for the Jeffrey Modell Foundation.